3 Basal Ganglia Group of deep nucleiCaudate nucleusPutamenGlobus pallidusSubstantia nigradopamine-rich pars compactapars reticularisInputs: Corpus striatum (caudate nucleus and putamen) receives input from the cerebral cortex and the thalamusOutputs: projects by way of the thalamus to the cerebral cortex and then to the pyramidal system

8 Tic DisordersTics are repeated, intermittent movements that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movementTourette syndrome : Multiple motor and vocal ticsEtiologyPrimary:the vast majoritySecondary:Huntingtons, encephalitis, medication induced, carbon monoxide poisoning, neuroacantocytosis

9 Tics Diagnosis: Associated with ADHD and OCD PANDAS TreatmentTypical movementsDon’t occur in sleepPatient usually unaware of it occurringPatient can usually suppress for a short timeBut when they do, it is accompanied by a discomfort and a strong urge to do the tic (a compulsion)Wax and wane over timeWorsen with stressAssociated with ADHD and OCDMake sure to ask both of patient and family historyPANDASTreatmentReassuranceTics tend to wax and wane; most children outgrow themMedications (when necessary)Stimulants bring out tics; if the have ADHD, they can’t use stimulantsTenexRisperidone

15 Classification of DystoniaBy locationGeneralized dystonia affects most or all of the body.Focal dystonia is localized to a specific part of the body.Blepharospasm, Cervical Dystonia, Task Specific Dystonia (eg Writers cramp)Multifocal dystonia involves two or more unrelated body parts.Segmental dystonia affects two or more adjacent parts of the body.Hemidystonia involves the arm and leg on the same side of the body.By etiologyPrimary: by definition, no other neurologic impairmentSecondary: Cerebral Palsy the most common cause in childrenPsychogenic

16 Primary Dystonias Genetic Dystonias DYT1 dystonia dominantly inherited generalized dystoniatypically begins in childhood, affects the limbs first, and progressesA great deal of phenotypic variabilityDopa-responsive dystonia (Segawa’s disease)onset during childhood and have progressive difficulty with walking. Symptoms characteristically fluctuate and are worse late in the day and after exercise. Some forms are due to mutations in the DYT5 gene for GTP cyclohydrolase 1. Patients with this disorder have dramatic improvements in symptoms after treatment with levodopaMany other genes that cause dystonic syndromes have been found

21 SterotypiesRepetitive, simple movements that can be voluntarily suppressed.Examples include repetitive chewing, rocking, twirling, or touching movementsMost common in children with autism or mental retardation; can occur in otherwise normal children.

23 Chorea, athetosis and ballismusan irregular, rapid, uncontrolled, involuntary, excessive movement that seems to flow randomly from one part of the body to another.The affected child often appears fidgety or restless and can’t sit stillAthetosisA slower writhing and twisting movement.Ballism (ballismus)chorea that affects proximal joints such as shoulder or hip, leading to large amplitude flailing movements of the limbs

26 Sydenham choreaSydenham chorea is a movement disorder characterized by chorea, emotional lability, and hypotonia. It is one of the major clinical manifestations of acute rheumatic fever (ARF).Symptoms of SC usually begin one to eight months after the onset of ARF. The symptoms typically improve gradually, with a mean duration of 12 to 15 weeks (At least 30 percent of individuals have clinical carditis in association with SC.DiagnosisThe diagnosis of SC is made clinically, based on characteristic neurological findings and a careful cardiac examination. If carditis is present, this confirms the diagnosis.The antistreptolysin O (ASLO) titer is of limited use in patients with SC, because titers generally peak before the onset of SC symptoms and children without rheumatic fever or SC often have low positive titers of ASLO.The antideoxyribonuclease (anti-DNAse) B titer is more useful for supporting the diagnosis of SC because it tends to remain elevated longer.If not clinicually definite, other causes of chorea should be excluded, including systemic lupus erythematosus, Huntington’s disease, and Wilson’s disease.TreatmentMost patients with SC recover fully without treatment, with symptoms lasting from a few weeks to one year or more.For those with significant impairment of motor function and the possibility of self injury consider corticosteroids (prednisone 1 mg/kg daily for two weeks and then tapered over two to three weeks)Valproic acid if needed to treat choreaUp to 30 percent of individuals with SC experience a recurrence, usually within a few years of the initial episode. The risk is probably reduced, by chronic treatment with prophylactic antibiotics.

30 Treatment of Chorea May be difficult to treat.Taper or discontinue any medications that can cause or worsen choreaIn adults, the mainstay of treatment in adults is neuroleptics, including haloperidol and pimozide.In children the incidence of side effects in children is high.Therefore, treatment is usuallyBenzodiazepine, particularly clonazepam, diazepam, or clobazamValproate, especially in Sydenham's chorea.Sydenham's choreaThere is considerable debate about whether children with Sydenham's chorea due to streptococcal infection should be given long-term antibiotics. There is not yet scientific evidence to support this, although short-term treatment is certainly needed in order to prevent complications such as rheumatic fever.

34 Essential Tremor Tremor should be the only neurologic manifestationUsually benign, but may progress to a disabling movement disorder.Hereditary ET can begin in infancyhereditary chin tremor and shuddering attacks.

35 Work up of TremorAny medications that may worsen tremor should be avoided, if possible.Check electrolytes, including glucose, calcium and magnesium, thyroid function, copper in the urine (for Wilson's disease), and possibly the amount of adrenaline metabolites (for pheochromocytoma).Consider MRI if the tremor had sudden onset,Consider EEG if there is suspicion for seizures.If parkinsonian features are present, consider a trial of L-DOPAIf there is a family history of tremor, it may be helpful to of alcohol ( in the affected family member). This suggests essential tremor.

37 Myoclonus Sudden, brief, jerky, shock-like involuntary movements.May be triggered by attempts at voluntary movement, sensory stimulation or startleMyoclonus is not suppressible and is often activated by volitional movement.Negative myoclonus is a sudden involuntary relaxation of a muscle, rather than a contraction.Myoclonus is often associated with epilepsy.